Retinoblastoma overview
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
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Overview
Retinoblastoma is a cancer of the retina. Development of this tumor is initiated by mutations[1] that inactivate both copies of the RB1 gene, which codes for the retinoblastoma protein.[2] It is a primitive neuroepithelial neoplasm. Retnal photoreceptor cells are developed from immature neural epithelium from which retinoblastoma is thought to arise from.[3] It is also known as Retinal Neuroblastoma, Retinal Glioma and Retinal Glioblastoma.
It occurs mostly in children younger than 5 years and accounts for about 3% of the cancers occurring in children younger than 15 years. Adult cases have also been clinically recorded.[4] The estimated annual incidence is approximately 4 per million children.[5] It begins with white blotches in one or both eyes (leukocoria) which can be seen in photographs (this is distinct from the red-eye effect which is normal); or when light reflects off the eye, as when watching television.
The tumor may begin in one or both eyes. Retinoblastoma is usually confined to the eye but can spread to the brain via the optic nerve.
As the retina is the light-sensitive part of the eye necessary for vision, loss of vision occurs.
The condition reached the headlines in May 2007, when Utah Jazz point guard Derek Fisher revealed that his 10-month-old daughter had the condition. Fisher missed Game 1 and half of Game 2 of the 2007 NBA playoff series versus the Golden State Warriors to be with his daughter for her surgery in New York City.
Trilateral Retinoblastoma
Trilateral retinoblastoma is the term that refers to bilateral retinoblastoma associated with an intracranial primitive neuroectodermal tumor in the pineal or suprasellar region. It has a poor prognosis especially when there is leptomeningeal involvement and when the tumor spreads in to subarachnoid space. For dissemination of tumor cells into the sub-arachnoid space, Optic nerve involvement is an important portal.[6] The mean length of survival is 9.7 months in patients who undergo treatment and 1.3 months in those who do not after a pineal or sellar mass is detected.[7]
References
- ↑ Knudson A (1971). "Mutation and cancer: statistical study of retinoblastoma". Proc Natl Acad Sci U S A. 68 (4): 820–3. PMID 5279gadgqetqer523 Check
|pmid=value (help). - ↑ Friend S, Bernards R, Rogelj S, Weinberg R, Rapaport J, Albert D, Dryja T. "A human DNA segment with properties of the gene that predisposes to retinoblastoma and osteosarcoma". Nature. 323 (6089): 643–6. PMID 2877398.
- ↑ Smirniotopoulos JG, Bargallo N, Mafee MF (1994). "Differential diagnosis of leukokoria: radiologic-pathologic correlation". Radiographics : a Review Publication of the Radiological Society of North America, Inc. 14 (5): 1059–79, quiz 1081–2. PMID 7991814. Retrieved 2012-05-02. Unknown parameter
|month=ignored (help) - ↑ Takahashi T, Tamura S, Inoue M, Isayama Y, Sashikata T (1983). "Retinoblastoma in a 26-year-old adult". Ophthalmology. 90 (2): 179–83. PMID 6856254.
- ↑ cancer.org
- ↑ Provenzale JM, Weber AL, Klintworth GK, McLendon RE (1995). "Radiologic-pathologic correlation. Bilateral retinoblastoma with coexistent pinealoblastoma (trilateral retinoblastoma)". AJNR. American Journal of Neuroradiology. 16 (1): 157–65. PMID 7900586. Retrieved 2012-05-02. Unknown parameter
|month=ignored (help) - ↑ Holladay DA, Holladay A, Montebello JF, Redmond KP (1991). "Clinical presentation, treatment, and outcome of trilateral retinoblastoma". Cancer. 67 (3): 710–5. PMID 1985763. Unknown parameter
|month=ignored (help);|access-date=requires|url=(help)